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Journal of Endocrinological Investigation

, Volume 16, Issue 4, pp 295–296 | Cite as

Multiple endocrine neoplasia type 2A and cutaneous lichen amyloidosis: Description of a new family

  • Furio Pacini
  • L. Fugazzola
  • G. Bevilacqua
  • P. Viacava
  • V. Nardini
  • E. Martino
Case Report

Abstract

Multiple endocrine neoplasia (MEN) 2A associated to cutaneous lichen amyloidosis is a variant of MEN 2A recently reported in few families. We describe an additional family with this syndrome. The skin lesion is a pruritic one, located over the upper back showing, at biopsy, cutaneous amyloid. The propositus of our family was a 35-year-old woman already treated with total thyroidectomy for medullary thyroid cancer and with bilateral adrenalectomy for pheochromocytoma. The patient referred that the skin lesion was present since childhood and increased with time. Skin biopsy showed negative staining for amyloid, for calcitonin and neuron-specific enolase. The paraspinal muscles corresponding to the area of affected skin showed slight neurogenic abnormalities. Ten other members of her family were affected by MEN 2A, three of whom (all females) had the same cutaneous alteration.

Key-words

Multiple endocrine neoplasia medullary thyroid cancer pheochromocytoma cutaneous lichen amyloidosis amyloid 

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Copyright information

© Italian Society of Endocrinology (SIE) 1993

Authors and Affiliations

  • Furio Pacini
    • 1
  • L. Fugazzola
    • 1
  • G. Bevilacqua
    • 2
  • P. Viacava
    • 2
  • V. Nardini
    • 2
  • E. Martino
    • 1
  1. 1.Istituto di EndocrinologiaUniversità di PisaTirrenia PisaItaly
  2. 2.Istituto di Anatomia PatologicaUniversità di PisaPisaItaly

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